Congenital Aplasia of the Lacrimal Gland Co-occurrent with Congenital Aplasia

Congenital aplasia of the lacrimal gland co-occurrent with congenital aplasia of the salivary glands means that a baby is born without the normal tear-making glands (lacrimal glands) and without some or all of the big saliva-making glands (major salivary glands). [1] This is usually called aplasia of lacrimal and salivary glands (ALSG). It is a very rare condition and most cases are genetic (passed in families) in an autosomal-dominant way, often linked to changes (mutations) in a gene called FGF10. 1

Congenital aplasia of the lacrimal gland co-occurrent with congenital aplasia of the salivary glands is usually part of a rare syndrome called “aplasia of lacrimal and salivary glands (ALSG).” In this condition, the tear (lacrimal) and major salivary glands do not form properly before birth, so the eyes produce very few tears and the mouth has very little saliva. This causes lifelong dry eye and dry mouth, with higher risk of eye infection, corneal damage, tooth decay, gum disease and oral infections. [1]

How this condition affects eyes and mouth

Because the lacrimal glands are absent or very small, the eye surface is not washed and nourished by normal tears. This can lead to burning, redness, light sensitivity, blurred vision and, in severe cases, corneal ulcer or scarring if not treated. At the same time, salivary gland aplasia means saliva is very low or absent, so food sticks to teeth, speech and swallowing are difficult, and cavities, oral thrush and periodontal disease are common without careful care. [2]

Because the lacrimal glands are missing or very small, the eyes do not make normal tears. The white part of the eye can be dry, red, and easily irritated. Children may have burning, gritty, or painful eyes and may also have odd watery eyes (tears running out all the time) because the surface is unhealthy. 2

Because the major salivary glands (parotid, submandibular, sublingual) are missing or very small, the mouth is very dry (xerostomia). This dryness makes it hard to chew, swallow, and speak. It also greatly increases the risk of tooth decay, gum disease, mouth infections, and bad breath. 3

ALSG can appear alone or as part of a wider group of problems called the LADD (lacrimo-auriculo-dento-digital) spectrum, where people can also have changes of the ears, teeth, and fingers. In many families, ALSG and LADD seem to be different faces of the same genetic problem involving FGF10 and related pathways. 4

Even though this condition is present from birth, it may not be noticed right away. Parents or doctors often start to worry because the child always has red, uncomfortable eyes and a very dry mouth, plus many dental problems very early in life. Early diagnosis is important because correct eye and dental care can prevent long-term damage. 5

Other names

Other names (synonyms) often used for this condition include:

1. Aplasia of lacrimal and salivary glands (ALSG) – the most common name in medical books. 1

2. ALSG syndrome – same condition, with “syndrome” added because more than one body system can be affected. 4

3. Lacrimal and salivary gland agenesis – “agenesis” also means glands did not form at all. 6

4. Congenital lacrimal and salivary gland aplasia – stresses that the child is born with the problem. 7

5. Lacrimal-salivary gland aplasia in the FGF10 spectrum – used in research about the FGF10 gene. 8

Types 

(There is no single “official” type system, but doctors often think in these groups):

1. Isolated ALSG – only the lacrimal and salivary glands are absent or very small; other body parts look normal. 3

2. ALSG within LADD spectrum – lacrimal and salivary aplasia plus ear changes, unusual teeth, or finger/toe differences. 4

3. Complete gland aplasia – all main lacrimal glands and all major salivary glands are missing. 9

4. Partial gland aplasia / hypoplasia – some glands are missing and others are present but too small or weak (hypoplastic). 10

5. Unilateral ALSG – glands missing on one side only (right or left). 6

6. Bilateral ALSG – glands missing on both sides; this is the most common pattern. 7

7. Familial ALSG – several family members are affected, usually in an autosomal-dominant pattern. 8

8. Sporadic ALSG – only one person in the family is affected; may be due to a new gene change (de novo mutation). 11

Each type helps doctors guess the underlying gene change, the risk for other family members, and the best plan for follow-up and screening. 5

Causes

1. FGF10 gene mutations
   The main known cause is a harmful change (mutation) in the FGF10 gene. This gene helps guide the growth of many glands and facial parts in the embryo. When FGF10 does not work properly, the lacrimal and salivary glands may never form. 8

2. Autosomal-dominant inheritance
   ALSG is often passed in families in an autosomal-dominant way. This means that if a parent has the condition or the gene mutation, each child has a 50% chance of inheriting it. 3

3. LADD syndrome–related variants
   Some people with FGF10 changes have not only ALSG but also ear, teeth, and finger changes (LADD). These gene variants sit in the same pathway and can cause a range from pure ALSG to full LADD syndrome. 4

4. FGFR2 and other receptor gene changes
   In some craniofacial syndromes, changes in genes like FGFR2 and related receptors disturb FGF signaling. This can alter the early development of glands and may be part of the wider FGF10-related spectrum. 12

5. De novo (new) mutations
   In some affected children, no other family member has the condition. In these cases, the gene change may have arisen for the first time in the egg, sperm, or very early embryo. 11

6. Incomplete gland budding in early embryo
   During early pregnancy, tiny buds grow out from the lining of the mouth and eye area to form glands. If these buds do not form or stop growing too soon, complete or partial aplasia can result. 13

7. Defects in ectodermal development
   The lacrimal and salivary glands come from the ectoderm (outer layer of the embryo). Many ectodermal disorders and syndromes have been linked with salivary aplasia and lacrimal problems, suggesting that broad ectoderm development errors can be a cause. 14

8. Association with craniofacial syndromes
   Conditions like mandibulofacial dysostosis and other craniofacial syndromes have been reported together with salivary gland aplasia. In these cases, the jaw and face shape problems and the gland problems come from shared early developmental pathways. 15

9. Association with hereditary ectodermal dysplasia
   Hereditary ectodermal dysplasia affects teeth, hair, nails, and glands. Salivary aplasia and lacrimal abnormalities are described in some patients, suggesting that the same gene errors that harm teeth and hair can also prevent gland formation. 14

10. Chromosome microdeletions including the FGF10 region
    In some rare patients, a small missing piece of chromosome that includes the FGF10 area may be found. This kind of structural change can remove the gene or its control regions and cause ALSG. 16

11. Modifier genes that change FGF10 signaling
    Some people with the same FGF10 mutation have milder or more severe disease. This suggests other genes modify how strongly the FGF10 pathway works, and these modifier genes can act as additional “causes” of how bad the aplasia becomes. 12

12. Embryonic blood supply problems to developing glands
    If the tiny blood vessels that should feed the early gland buds do not form well, the buds may fail to grow. This idea is used to explain some isolated gland aplasia cases, though direct proof is limited. 9

13. Shared developmental field disturbance of orbit and oral region
    The eyes and mouth form from nearby facial regions. A defect in this shared developmental field may lead to combined eye gland and mouth gland absence, as seen in ALSG and related disorders. 5

14. Rare environmental factors in early pregnancy (theoretical)
    Severe infections, toxins, or certain drugs in early pregnancy can disturb craniofacial development in general. Although there is no strong proof for a specific environmental cause for ALSG, such exposures might modify risk in people who already carry a gene change. 13

15. Advanced parental age (possible risk factor)
    Some genetic disorders with de novo mutations are more common with older paternal age. This pattern has not been firmly proven for ALSG, but it is sometimes considered when a child is affected and both parents are clinically normal. 11

16. Undetected mosaicism in a parent
    A parent may carry the FGF10 mutation in only some cells (mosaicism) and look normal, yet still pass it to a child. In that child, the mutation is present in all cells and causes full ALSG. 8

17. Epigenetic changes affecting gland genes
    Epigenetic marks are chemical tags on DNA that turn genes on or off. Abnormal epigenetic control of gland-development genes may contribute to gland aplasia in some individuals, though evidence is still limited and mostly based on general developmental biology. 12

18. Overlap with other FGF10-related disorders
    FGF10 changes can also cause lung and airway problems in some children. These overlapping FGF10-related disorders show that the same gene is vital for many organs, and differences in when and where the gene fails can explain why some people have ALSG and others do not. 16

19. Unknown genetic factors
    Some people with clear ALSG have no detectable FGF10 mutation on standard tests. This suggests that other, still-unknown genes that interact with the FGF pathway may be responsible. 11

20. Unknown / idiopathic causes
    In a few patients, no family pattern, no obvious gene change, and no clear environmental factor can be found. In these cases the cause is called “idiopathic,” meaning that current medical science does not yet know the reason. 3

Symptoms and signs

1. Dry, irritated eyes (xerophthalmia)
   The eyes feel dry, burning, or gritty because there are not enough tears to keep the surface moist. Redness, discomfort, and sensitivity to light are common, even in young children. 2

2. Frequent eye infections
   Tears help wash away germs. Without them, bacteria and viruses can stay on the eye surface. This leads to repeated conjunctivitis and sometimes more serious infections of the cornea. 7

3. Abnormal watering of the eyes (epiphora)
   Even though tear production is low, the eye surface is so unhealthy that any small amount of fluid or reflex tears may spill over the eyelid. This can look like “constant tearing” even in a dry eye condition. 2

4. Eye pain and foreign-body sensation
   Many patients describe the feeling that “sand” or a “foreign body” is in the eye, especially in wind, air-conditioning, or during reading. This comes from small defects in the corneal surface. 6

5. Conjunctival scarring over time
   Long-standing dryness can damage the delicate lining of the eye (conjunctiva), causing scarring. This can change how the eyelids move over the eye and make dryness even worse. 1

6. Very dry mouth (xerostomia)
   Because salivary glands are absent, the mouth is persistently dry. The tongue may stick to the roof of the mouth, and the person may need to sip water often, especially during meals or talking. 3

7. Problems chewing, swallowing, and speaking
   Saliva helps break down food and makes swallowing smooth. Without saliva, dry food is hard to chew and swallow, and speech may become unclear or tiring, especially after talking for some time. 10

8. Early and severe dental caries (tooth decay)
   Saliva neutralizes acids and washes away food particles. In ALSG, lack of saliva means acids from bacteria stay on teeth, causing very early and often severe tooth decay in children. 14

9. Gum disease and tooth loss
   Dryness and chronic bacterial growth around the gums lead to gingivitis and later periodontitis. Without good care, this can cause loosening and loss of teeth. 3

10. Recurrent mouth and throat infections
    Patients often get mouth ulcers, fungal infections like oral thrush, and infections of the upper airway, such as pharyngitis or laryngitis, because saliva’s protective action is missing. 14

11. Halitosis (bad breath)
    Food debris and bacteria remain in the dry mouth for longer. They produce sulfur compounds and bad smells, leading to persistent bad breath even with brushing. 8

12. Difficulty wearing contact lenses
    In older children or adults, contact lenses may feel very uncomfortable or impossible to wear because of the lack of tears and the fragile corneal surface. 6

13. Eye fatigue and blurred vision
    The cornea must be smooth and wet to focus light well. Dryness can cause variable blurred vision, especially later in the day, and can make reading or screen work tiring. 7

14. Speech changes (hoarse or rough voice)
    Chronic dryness in the throat and larynx and frequent infections can make the voice sound hoarse or rough, especially after talking for a while or during infections. 14

15. Possible associated features (ears, teeth, fingers) in LADD spectrum
    In some people, especially with LADD-type disease, there may be low-set or malformed ears, missing or small teeth, or unusual fingers and toes. These extra features help doctors suspect a syndromic form related to FGF10. 4

Diagnostic tests

Physical exam

1. Detailed medical and family history
   The doctor asks about eye dryness, mouth dryness, dental problems, and infections from early childhood. They also ask if other family members have similar issues, which can suggest a genetic cause like ALSG. 1

2. General physical examination
   The doctor looks at the whole body to find signs of a syndrome, such as unusual facial shape, ear changes, or finger differences. These clues help decide if the condition is isolated ALSG or part of LADD or another syndrome. 4

3. External eye and eyelid examination
   Using a light and magnifier (slit lamp), the eye doctor examines the eyelids, the eyelid margin, and the conjunctiva for redness, scarring, and signs of severe dryness. They also look for missing lacrimal puncta (the small tear drainage openings). 2

4. Oral cavity examination
   The mouth, tongue, and throat are checked for dryness, cracks, ulcers, and redness. The doctor looks at the amount and thickness of saliva on the tongue and inside the cheeks. 10

5. Dental and gum examination
   A dentist examines teeth for early cavities, erosion, and plaque build-up, and checks the gums for swelling or bleeding. Severe caries in a very young child with dry mouth strongly supports major salivary gland aplasia. 14

Manual tests

6. Schirmer tear test
   A small paper strip is placed gently under the lower eyelid for a few minutes to measure how much it becomes wet. Very low wetting shows reduced tear production and supports lacrimal gland aplasia or hypoplasia. 6

7. Tear film break-up time (TBUT)
   A dye called fluorescein is put into the eye. The doctor shines a blue light and measures how fast dry spots appear on the cornea. A very short break-up time means an unstable tear film and severe dryness. 7

8. Palpation of lacrimal gland area
   The doctor gently presses the area under the outer upper eyelid where the lacrimal gland should be. In normal people, a soft mass may be felt. In aplasia, there is often no palpable gland tissue. 2

9. Palpation and stimulation of major salivary glands
   The doctor feels the areas over the parotid, submandibular, and sublingual glands and tries to “milk” saliva from the ducts by pressing. In gland aplasia, there is usually no or very little saliva flow and sometimes no duct opening can be seen. 9

Lab and pathological tests

10. Basic blood tests and inflammatory markers
    Routine blood tests and markers like ESR and CRP can help rule out other causes of eye and mouth dryness, such as autoimmune diseases or chronic infections. Normal results support a primary developmental problem like ALSG. 3

11. Autoantibody tests (for Sjögren and other autoimmune diseases)
    Tests for antibodies such as anti-SSA/Ro and anti-SSB/La are often done to exclude Sjögren syndrome, a common cause of dry eye and dry mouth in adults. In congenital ALSG, these tests are usually negative. 3

12. Genetic testing for FGF10 and related genes
    A blood sample can be used to read the DNA sequence of FGF10 and sometimes FGFR2 or other related genes. Finding a disease-causing mutation confirms the diagnosis of ALSG and allows family counseling. 8

13. Saliva flow measurement (sialometry)
    The patient is asked to spit into a tube over a set time, sometimes with and without stimulation (for example, lemon juice). Very low saliva volume supports a diagnosis of major salivary gland aplasia or severe dysfunction. 15

14. Saliva composition tests and microbiology
    If some saliva is present, its composition, pH, and bacterial content may be studied. Recurrent fungal or bacterial overgrowth is common in people with chronic dry mouth from gland aplasia. 10

Electrodiagnostic tests

15. Facial nerve conduction studies
    Electrodes are placed on the skin to measure how well the facial nerve carries signals to the facial muscles. This test is mainly used to rule out nerve damage as a cause of poor gland function; in pure ALSG, nerve conduction is usually normal. 13

16. Electromyography (EMG) of facial muscles
    EMG uses fine needles or surface electrodes to record electrical activity in muscles. Normal EMG with severe dryness suggests that the problem lies in missing glands rather than in weak facial muscles. 12

17. Autonomic function tests related to tear and saliva production (specialized)
    In some research centers, special tests measure how the autonomic nervous system controls tear and saliva flow. In ALSG, these reflexes may try to work but cannot produce normal fluid because the glands are absent. 16

Imaging tests

18. Orbital MRI or CT to view lacrimal glands
    MRI or CT scans of the eye sockets show whether lacrimal glands are present, small, or missing. In congenital lacrimal gland aplasia, this imaging typically shows complete absence or marked under-development of the glands. 6

19. Ultrasound of salivary glands
    Ultrasound uses sound waves to create images of the parotid, submandibular, and sublingual glands. In aplasia, the glands cannot be seen in their usual positions, or only small remnants are visible. 9

20. CT or MRI of salivary glands and surrounding structures
    CT or MRI scans of the face and neck give a detailed picture of where the salivary glands should be. Absence of the glands on both sides, together with clinical dryness, strongly supports the diagnosis of congenital salivary gland aplasia. 10

21. Sialography (contrast imaging of salivary ducts)
    In some cases, a contrast dye is injected into the salivary duct and X-rays are taken. If the duct system is very small or does not fill at all, this supports the idea that the gland tissue is missing or extremely reduced. 15

22. Dacryocystography or dacryoscintigraphy (tear drainage imaging)
    Contrast dye or a radioactive tracer is placed in the eye to follow the tear drainage system. These tests help separate drainage problems from tear-production problems and may show other lacrimal system defects that occur together with ALSG. 18

Non-pharmacological treatments (Therapies and others)

1. Frequent preservative-free lubricating eye drops
Using preservative-free artificial tears many times a day is the foundation of eye care. These drops replace the missing watery part of tears and help the eyelids glide smoothly over the cornea. Preservative-free products are safer for life-long use and for very dry eyes because preservatives can damage the surface over time. Regular use can reduce burning, foreign-body sensation and fluctuating vision. [4]

2. Lubricating eye gels and ointments at night
Thicker gels and ointments stay on the eye surface longer than drops, especially during sleep when blinking stops. They form a protective coating that reduces overnight drying and helps the cornea heal tiny surface defects. Vision may be blurry immediately after putting them in, so they are usually used at bedtime. Good night-time lubrication can greatly reduce morning pain and redness. [5]

3. Punctal occlusion or cautery
Even with lacrimal aplasia, accessory glands and serum tears may still produce small amounts of fluid. Blocking the tear drainage openings (puncta) with plugs or cautery keeps limited tears and lubricants on the eye longer. Studies of congenital lacrimal gland agenesis show punctal occlusion, together with lubricants, can improve symptoms and corneal staining. This procedure is done in the clinic with local anesthesia. [6]

4. Scleral or PROSE® lenses
Large-diameter scleral lenses vault over the cornea and hold a reservoir of sterile fluid against the eye all day. This fluid bath protects the corneal surface and gives more stable vision. In severe dry eye from lacrimal gland agenesis, scleral systems (including PROSE® devices) have shown excellent symptom relief and healing of keratitis when standard drops are not enough. [7]

5. Humidifier and environmental control
Room humidifiers, especially in air-conditioned or heated homes, decrease evaporation of the tear film and oral moisture. Avoiding direct fans, smoke and wind, and taking breaks from digital screens (which reduce blink rate) also help. Simple changes, like wearing wrap-around glasses outdoors, can significantly reduce eye dryness and irritation. [8]

6. Frequent water sipping and sugar-free chewing gum
For xerostomia, taking sips of water throughout the day and chewing sugar-free gum or sucking sugar-free lozenges stimulates any remaining minor salivary glands. Xylitol-containing gums can also reduce cavity-causing bacteria. These simple measures can ease speaking and swallowing and are strongly recommended in xerostomia guidelines. [9]

7. Saliva substitute gels and sprays
Commercial saliva substitutes (gels, sprays or rinses) mimic the slipperiness and pH of saliva. They do not replace all saliva functions but help reduce friction, mouth sores and difficulty with dentures. Many formulations contain carboxymethylcellulose or similar polymers to hold moisture on oral surfaces. Regular use before meals, speech or bedtime can improve comfort. [10]

8. Intensive fluoride and remineralizing care
Because saliva protects teeth, xerostomia greatly increases caries risk. Daily use of high-fluoride toothpaste or gel, fluoride varnish applications, and, for high-risk patients, custom trays with 1.1% sodium fluoride gel, are recommended to prevent decay. Evidence shows that combining good oral hygiene with topical fluoride can significantly reduce xerostomia-related caries. [11]

9. Regular dental care and early treatment of oral disease
People with salivary gland aplasia need very frequent dental check-ups (often every 3–4 months). Dentists monitor for new cavities, gum inflammation and fungal infections and can treat problems early with fillings, sealants, periodontal therapy or antifungals. Close collaboration between dentist and physician is essential to maintain oral function and prevent tooth loss. [12]

10. Bland, non-alcohol mouth rinses
Alcohol-containing mouthwashes make xerostomia worse. Neutral-pH, alcohol-free rinses or simple salt-bicarbonate solutions can freshen the mouth, reduce irritation and help clear food debris without drying. Some rinses also include fluoride, calcium or phosphate to support remineralization. [13]

11. Diet texture modification
Soft, moist foods with added gravies, sauces or broths are easier to chew and swallow with a dry mouth. Avoiding very dry crackers or chips and alternating bites of food with sips of water helps prevent choking and improves enjoyment of meals. For children, nutritionists can design calorie-dense, moist recipes to support normal growth. [14]

12. Protection from vitamin A deficiency and malnutrition
If diet is poor, vitamin A deficiency can worsen eye dryness and corneal damage. Ensuring enough vitamin A through foods (eggs, dairy, leafy greens, orange fruits) or supervised supplements is important, especially in low-resource settings, to prevent xerophthalmia and potential blindness. [15]

13. Eye protection outdoors (wrap-around glasses)
Wrap-around sunglasses shield the eyes from wind, dust and UV light, which all increase evaporation. They also reduce light sensitivity, a common complaint in severe dry eye. Simple physical barriers like side shields can noticeably improve comfort during outdoor activities. [16]

14. Eyelid hygiene and warm compresses
Regular warm compresses and gentle lid cleaning support the meibomian glands, which produce the oily layer of the tear film. This oily layer slows evaporation. In dry eye disease and meibomian gland dysfunction, lid hygiene improves tear stability and can reduce irritation, so it is helpful even when lacrimal glands are absent. [17]

15. Avoidance of drying medications and substances
Some drugs (antihistamines, certain antidepressants, anticholinergics) and habits (smoking, heavy caffeine or alcohol use) worsen dryness. Reviewing all medicines with a doctor and pharmacist, and avoiding cigarettes and excess caffeine, can reduce symptoms and improve effect of other treatments. [18]

16. Speech and swallowing therapy
Speech-language therapists can teach strategies to manage speaking, chewing and swallowing with a dry mouth, such as pacing, safe swallowing techniques and posture adjustments. This is especially important in children, who may struggle in school or social situations because of discomfort or difficulty speaking clearly. [19]

17. Psychological and social support
Living with a rare visible condition, frequent dental work and chronic eye discomfort can affect mood, school or work, and self-esteem. Counseling and patient support groups help families understand the condition, cope with anxiety or sadness and learn from others’ experiences. [20]

18. School and workplace accommodations
Extra water breaks, permission to use eye drops in class, seating away from air vents and flexible time for dental or eye appointments can make a big difference. Clear medical letters explaining the condition and its needs help teachers and employers support the person appropriately. [21]

19. Regular ophthalmology monitoring
Scheduled eye exams with careful corneal staining, tear tests and imaging help detect early damage. Long-term follow-up is vital because the cornea can deteriorate silently until vision is suddenly threatened. Early adjustments in therapy can often prevent severe complications. [22]

20. Genetic counseling for the family
ALSG is usually autosomal dominant, meaning it can pass from an affected parent to a child. Genetic counseling explains inheritance, recurrence risks and options for family planning. It also helps relatives understand what symptoms to watch for and when to seek evaluation. [23]

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Drug treatments

Important: Drug names, doses and times below are from regulatory labels and studies. They are examples, not personal medical advice. Dosing must always be decided by a qualified doctor for each patient.

1. Pilocarpine (Salagen) – oral sialogogue
Pilocarpine is a cholinergic agonist that stimulates muscarinic receptors in residual salivary tissue, increasing saliva production and easing dry mouth and swallowing problems. FDA labeling for Salagen shows benefit in xerostomia due to Sjögren’s syndrome and radiation. Typical adult doses are 5 mg orally three or four times daily, taken with water. Common side effects include sweating, flushing and increased urination; caution is needed in asthma and heart disease. [24]

2. Cevimeline (Evoxac) – oral sialogogue
Cevimeline is another muscarinic agonist that increases exocrine gland secretion. FDA reviews indicate its approval for xerostomia in Sjögren’s syndrome. The usual adult dose is 30 mg by mouth three times daily. It can improve mouth wetness, chewing and speaking but may cause sweating, nausea, visual changes or heart rhythm issues, so it is contraindicated in uncontrolled asthma and angle-closure glaucoma. [25] [26]

3. Artificial tear solutions (carboxymethylcellulose / HPMC-based)
Many over-the-counter lubricating drops use polymers such as carboxymethylcellulose or hydroxypropyl methylcellulose to hold water on the eye surface. FDA monographs on ophthalmic drug products describe directions like 1–2 drops in affected eyes as needed. These products improve comfort and tear stability, with minimal systemic side effects, and are safe for frequent long-term use when preservative-free. [27]

4. Lubricating eye ointments (OTC petrolatum-based)
Night-time lubricating ointments that combine mineral oil and petrolatum form a thick protective film on the cornea, reducing dryness and exposure keratopathy. OTC labeling usually advises a small ribbon inside the lower lid at bedtime. Blurred vision shortly after use is expected. These products are helpful in severe cases where drops alone cannot maintain surface moisture overnight. [28]

5. Cyclosporine ophthalmic emulsion 0.05% (Restasis)
Cyclosporine eye drops are FDA-approved to increase tear production in dry eye linked to ocular surface inflammation. The label recommends one drop in each eye twice daily, 12 hours apart. Cyclosporine acts as an immunomodulator, reducing T-cell–mediated inflammation so remaining tear-producing cells work better. Burning on instillation is common, but serious systemic side effects are rare because absorption is minimal. [29]

6. Lifitegrast 5% ophthalmic solution (Xiidra)
Lifitegrast is an LFA-1 antagonist approved to treat signs and symptoms of dry eye disease. The label advises one drop in each eye twice daily, about 12 hours apart. It blocks interaction between LFA-1 and ICAM-1, reducing T-cell activation and ocular surface inflammation. Trials show improvement in symptoms and some signs by 84 days, with side effects such as eye irritation, altered taste and reduced visual acuity in some patients. [30]

7. Higher-strength cyclosporine ophthalmic solutions (e.g., Cequa 0.09%)
Newer formulations of cyclosporine ophthalmic solution at higher concentrations have been approved to treat dry eye disease. Regulatory reviews note similar mechanisms (local immunomodulation and improved tear production) with different vehicles that may enhance corneal penetration. Dosing is generally one drop twice daily. These options may be considered when standard cyclosporine emulsion is insufficient. [31]

8. Topical NSAID eye drops (e.g., ketorolac, ACUVAIL)
Short courses of NSAID eye drops can help control pain and inflammation after procedures or during acute flares. The ACUVAIL label, for example, recommends one drop twice daily around cataract surgery. In ALSG, they may be used briefly to calm surface inflammation but are not for long-term daily use because of risk of corneal toxicity. [32]

9. Short-course topical corticosteroid eye drops
Low-potency steroid drops (e.g., loteprednol or medrysone) can be used for short periods in severe keratitis to reduce inflammation and pain. FDA labels describe them as anti-inflammatory agents that inhibit multiple inflammatory pathways. Because steroids increase risk of infection, glaucoma and cataract, they must be used under specialist supervision with monitoring of eye pressure. [33]

10. Antifungal agents for oral candidiasis (e.g., nystatin suspension)
Dry mouth, poor clearance of food and frequent antibiotic use can promote oral thrush. Nystatin oral suspension swished and swallowed (or spat) several times a day is commonly used to treat candidiasis, reducing soreness, burning and difficulty eating. These drugs act locally on fungal cell membranes and are generally safe, but prescription and monitoring are required. [34]

11. High-fluoride toothpaste or gels (1.1% sodium fluoride)
Prescription-strength fluoride toothpaste or gels are recommended for patients with severe xerostomia to prevent cavities. Guidance from dental associations suggests daily use under dental supervision. Fluoride strengthens enamel, promotes remineralization and inhibits bacterial metabolism. Over-ingestion must be avoided, especially in young children, to prevent fluorosis. [35]

12. Antibacterial or antiseptic mouthrinses (non-alcohol, fluoride-containing)
Carefully selected antibacterial rinses can reduce plaque and gingivitis in xerostomia, but many standard alcohol-based formulas are too drying. Fluoride-containing anticavity rinses used once daily may be beneficial for high-risk patients when advised by a dentist. They act by lowering bacterial counts and enhancing enamel resistance to acid. [36]

13. Systemic omega-3 fatty acid supplements (supportive therapy)
Although not specific ALSG drugs, randomized trials in dry eye disease show oral omega-3 fatty acids can improve tear stability and symptoms in some patients. Typical study doses range around 1–3 g/day of EPA/DHA combinations. Omega-3s have anti-inflammatory effects on the ocular surface and meibomian glands, but results are mixed, so they are considered adjunctive rather than primary therapy. [37]

14. Vitamin A supplementation (when deficient)
In areas where vitamin A deficiency is present, supplementation is critical, because deficiency can cause xerophthalmia, corneal ulceration and blindness. Dosing regimens depend on age and severity and must follow WHO or national protocols. Vitamin A supports normal differentiation of ocular surface epithelium and tear production; correcting deficiency can reverse many early eye changes. [38]

15. Autologous serum eye drops (ASEDs)
ASEDs are prepared from the patient’s own blood and contain growth factors and vitamins similar to natural tears. Clinical trials show that ASEDs improve symptoms, tear stability and epithelial defects in severe dry eye compared with artificial tears. Typical protocols use 20–50% serum diluted in saline, applied several times daily, under strict sterile conditions. They are usually reserved for specialized centers. [39]

16. Platelet-rich plasma (PRP) eye drops
PRP eye drops are richer in growth factors than standard serum drops and are being studied for severe ocular surface disease. Early studies suggest benefits in epithelial healing and symptom relief, but access is limited and preparation standards vary. They are considered experimental and should only be used in research-level or highly specialized clinics. [40]

17. Topical Coenzyme Q10 eye preparations (experimental)
Topical CoQ10 has shown neuroprotective and anti-apoptotic effects in corneal and retinal cells in experimental models, improving healing and reducing oxidative stress. Some small studies suggest potential benefit in ocular surface disease, but these products are not standard for ALSG and remain investigational or off-label. [41]

18. Multi-ingredient ocular nutritional supplements
Clinical studies of supplements containing lutein, zeaxanthin, curcumin and vitamin D3 show improved dry eye symptoms and inflammatory markers in some patients. These products act through antioxidant and anti-inflammatory pathways and by supporting tear film and ocular surface health. They can be considered as adjuncts, not replacements, for core therapies. [42]

19. Systemic immunosuppressants (in syndromic autoimmune cases)
If ALSG is part of a broader autoimmune disease such as Sjögren’s syndrome, systemic immunosuppressants (e.g., hydroxychloroquine, methotrexate, biologics) may be used to treat the underlying disease, which can secondarily improve dry eye and mouth symptoms. These drugs act on immune pathways rather than directly on the glands and require specialist rheumatology supervision and blood monitoring. [43]

20. Pain control and neuropathic eye-pain medications
In some patients, ocular pain persists even when the surface looks improved (“neuropathic corneal pain”). Medications such as systemic gabapentinoids, tricyclic antidepressants or topical therapies may be used under pain-specialist or ophthalmologist guidance. They modulate nerve signaling but do not treat dryness itself, so they are usually combined with surface therapies. [44]

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Dietary molecular supplements

(All doses must be individualized by a clinician; typical ranges below come from dry eye and nutrition studies.)

1. Omega-3 fatty acids (EPA/DHA)
Omega-3 supplements (fish oil or algae-based) in doses around 1–3 g/day are widely studied in dry eye. They reduce inflammatory mediators, may improve meibomian gland function and increase tear stability in some trials. They also support cardiovascular health. Side effects include gastrointestinal upset and, rarely, bleeding risk at high doses or with anticoagulants, so medical advice is needed. [45]

2. Vitamin A (retinol or beta-carotene)
Vitamin A is essential for healthy ocular surface epithelium and normal tear production. In deficient individuals, supervised supplementation can reverse xerophthalmia and prevent corneal ulceration and blindness. Typical treatment doses follow WHO protocols and are much higher than standard multivitamin doses, so they must be given under medical supervision to avoid toxicity. [46]

3. Vitamin D3
Vitamin D has immunomodulatory and anti-inflammatory effects. Clinical and experimental work suggests that low vitamin D may be linked with more severe dry eye. Supplementation in deficient patients can support immune balance and may improve symptoms, though evidence is still developing. Common oral doses range from 800–2000 IU/day, adjusted to blood levels. [47]

4. Curcumin (turmeric extract)
Curcumin is a polyphenol with strong anti-inflammatory and antioxidant actions. Ophthalmic research suggests curcumin can reduce inflammatory cytokines and oxidative stress in ocular surface cells and may help in dry eye and allergic eye disease. Oral doses in studies vary (often 500–1000 mg/day), usually with absorption-enhancing formulations. It can interact with anticoagulants and should be used carefully in people with gallbladder disease. [48]

5. Coenzyme Q10 (CoQ10)
CoQ10 is a mitochondrial antioxidant that supports cellular energy and protects against oxidative damage. Ophthalmic studies show it can reduce apoptosis in corneal and retinal cells and may help in ocular surface and retinal diseases. Oral doses often range 100–200 mg/day. Side effects are usually mild (GI discomfort), but it can affect warfarin response. [49]

6. Lutein and zeaxanthin
These carotenoids accumulate in the macula and also have antioxidant effects on the ocular surface. Multi-ingredient supplement trials including lutein and zeaxanthin report improvements in dry eye symptoms and inflammatory markers. Typical doses are 10–20 mg lutein with 2 mg zeaxanthin daily. They are generally safe and commonly used in macular health formulas. [50]

7. Vitamin C (ascorbic acid)
Vitamin C supports collagen synthesis and acts as a water-soluble antioxidant in tears and saliva. Adequate intake (e.g., 100–500 mg/day dietary or supplemental) helps general tissue repair and immune function. It is not a specific dry eye drug but supports healing after corneal epithelial injury and protects against oxidative stress. [51]

8. Vitamin E (alpha-tocopherol)
Vitamin E is a fat-soluble antioxidant that protects cell membranes from oxidative damage. In some ocular studies, combinations of vitamin E with CoQ10 enhance neuroprotective effects. Usual oral doses range from 100–400 IU/day, but high doses may increase bleeding risk, especially with anticoagulants. [52]

9. Zinc
Zinc is important for immune function and epithelial repair. Deficiency can impair wound healing and taste, which may worsen eating problems in xerostomia. Oral supplements (often 10–25 mg elemental zinc/day) are given when deficiency is suspected, but excessive intake can cause copper deficiency, so monitoring is needed. [53]

10. Selenium
Selenium is a trace element that supports antioxidant enzymes such as glutathione peroxidase. It may help protect tissues from oxidative stress, including ocular and oral tissues. Supplemental doses typically range 50–200 µg/day, avoiding excess because very high intake is toxic. [54]

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Immunity-boosting / regenerative / stem-cell–related therapies

(Most of these are research or specialist treatments, not routine self-medication.)

1. Autologous serum eye drops (regenerative tear substitute)
ASEDs contain growth factors, vitamins and immunoglobulins similar to natural tears. They support regeneration of corneal epithelium, reduce inflammation and improve nerve health. Randomized trials show better symptom relief and tear stability compared with artificial tears in severe dry eye. Dosing is individualized (often 4–8 times/day). Preparation must follow strict blood-handling rules. [55]

2. Platelet-rich plasma eye drops
PRP eye drops are prepared from concentrated platelets and release high levels of growth factors such as PDGF and TGF-β. These factors can promote epithelial healing and nerve regeneration on the ocular surface. Early clinical studies suggest benefit in severe dry eye and persistent epithelial defects, but protocols and availability vary, so therapy remains specialized. [56]

3. Mesenchymal stem cell (MSC) therapy for dry eye
Recent randomized trials in Sjögren-related dry eye have injected allogeneic adipose-derived MSCs into the lacrimal gland, showing improvements in tear production, stability and symptoms over months. MSCs appear to modulate immune responses and support tissue repair. These treatments are still experimental, used only in controlled trials and not standard for ALSG. [57]

4. MSC-derived exosome eye drops
Animal and early human studies are exploring eye drops made from vesicles (exosomes) released by MSCs. These exosomes carry anti-inflammatory and regenerative signals that may improve dry eye signs without the safety concerns of live cell injection. Triple-blinded trials are underway in Sjögren’s dry eye. [58]

5. Topical Coenzyme Q10 and antioxidant eye drops
Topical CoQ10 formulations protect corneal and retinal cells from apoptosis and oxidative stress in experimental models and small clinical studies. By improving mitochondrial function and reducing free radicals, they may help preserve nerve and epithelial health in chronic dry eye. These products are still emerging and should be used only under specialist advice. [59]

6. Systemic immune-modulating biologics (for associated autoimmune disease)
In patients whose ALSG is part of systemic autoimmune disease, biologic agents (for example, B-cell or cytokine-targeting drugs) may reduce gland-directed immune attack, indirectly helping eye and mouth symptoms. These drugs act deeply on immune pathways and require rheumatology supervision, screening for infections and long-term monitoring. [60]

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Surgical and procedural options

1. Permanent punctal cautery
If temporary plugs help but fall out, surgeons can permanently close the puncta using cautery. This prevents drainage of any tears or lubricants, keeping moisture on the surface longer. It is considered in severe dry eye when other measures are not enough and when there is no need for tear drainage (for example, chronic tearing is not a problem). [61]

2. Tarsorrhaphy (partial eyelid closure)
In extreme cases with non-healing corneal ulcers, surgeons may stitch part of the eyelids together to narrow the opening. This reduces exposure, evaporation and mechanical trauma, allowing the cornea to heal. It can be temporary or permanent and is reserved for sight-threatening situations. [62]

3. Amniotic membrane transplantation
Amniotic membrane (from screened donors) can be placed on the cornea as a biological bandage when there are persistent epithelial defects or ulcers. It provides growth factors, reduces inflammation and encourages regeneration of healthy epithelium. It is often combined with intensive lubrication and protective lenses. [63]

4. Minor salivary gland transplantation to the eye (experimental)
In some specialized centers, small salivary glands from inside the lip have been transplanted to the conjunctival area to provide local fluid secretion to the eye surface. This complex microsurgery is considered only in very severe refractory cases and is still largely experimental. [64]

5. Dental restorations and prosthetic rehabilitation
Extensive cavities and tooth loss from xerostomia may require crowns, bridges, implants or dentures. Restorative dentistry is not just cosmetic: it restores chewing, nutrition and speech. Dentists often use materials and designs that tolerate a dry environment, combined with ongoing fluoride and hygiene measures. [65]

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Prevention and lifestyle

1. Maintain excellent oral hygiene (gentle brushing with fluoridated toothpaste twice daily and daily flossing). [66]

2. Visit the dentist and ophthalmologist regularly, even when symptoms feel “stable.” [67]

3. Avoid smoking and second-hand smoke, which irritate eyes and dry the mouth. [68]

4. Limit caffeine (strong tea, coffee, energy drinks) and alcohol, which increase dehydration. [69]

5. Use only alcohol-free, neutral-pH mouthrinses recommended by your dentist. [70]

6. Protect eyes from wind, dust and strong sunlight with wrap-around glasses. [71]

7. Keep indoor air humid and avoid strong direct air from fans or AC on the face. [72]

8. Review all medicines regularly with your doctor to minimize drugs that worsen dryness. [73]

9. Ensure adequate nutrition, including vitamin A–rich foods, and manage any malabsorption or systemic disease. [74]

10. Educate teachers, family and employers so they understand why water, eye drops and doctor visits are essential. [75]

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When to see a doctor urgently

You should see an eye doctor immediately if there is sudden vision loss, severe eye pain, marked redness, light sensitivity, or any sign of a corneal ulcer (white spot on the cornea, discharge, inability to open the eye). In people with ALSG, corneal damage can progress quickly because the surface has little natural protection. [76]

See a dentist or doctor promptly if you notice rapidly increasing cavities, broken teeth, painful mouth sores, white patches that do not wipe off (possible thrush), trouble swallowing, unexplained weight loss, or difficulty eating enough. Early treatment can prevent tooth loss and serious infections. [77]

Because this is a congenital, lifelong condition, regular planned follow-ups with ophthalmology, dentistry and sometimes genetics or rheumatology are just as important as emergency visits. [78]

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What to eat and what to avoid

1. Eat soft, moist foods with added sauces, gravies, soups or yogurt to make swallowing easier. [79]

2. Choose foods rich in vitamin A and antioxidants, such as carrots, sweet potatoes, spinach, eggs and dairy, if tolerated. [80]

3. Include healthy fats (olive oil, nuts, seeds, oily fish) to support absorption of fat-soluble vitamins and provide omega-3s. [81]

4. Drink water regularly in small sips; keep a bottle nearby instead of drinking large amounts at once. [82]

5. Use sugar-free chewing gum or xylitol lozenges after meals to stimulate any remaining saliva and reduce caries risk. [83]

6. Avoid very sugary snacks and drinks, which greatly increase cavity risk when saliva is low. [84]

7. Avoid very spicy, salty or acidic foods if they burn the mouth or worsen soreness. [85]

8. Limit hard, dry foods like crackers, toast and crisps that can be painful to chew and may injure fragile oral tissues. [86]

9. Avoid alcohol-containing drinks and mouthwashes because they further dry the mucosa. [87]

10. Work with a dietitian if weight loss, poor appetite or nutritional concerns appear; tailored meal plans can maintain growth and health. [88]

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Frequently asked questions (FAQs)

1. Is there a cure for congenital aplasia of lacrimal and salivary glands?
At present there is no way to “grow back” the missing glands. Treatment focuses on replacing tears and saliva, protecting eyes and teeth, and using supportive and regenerative therapies to preserve vision and oral function as much as possible. [89]

2. Is this condition genetic?
Yes, many patients have ALSG as an autosomal dominant disorder, meaning a change in a single gene copy can cause the condition. However, not every person in an affected family has the same severity; some may have milder gland underdevelopment rather than complete aplasia. [90]

3. Can children with this condition have normal vision?
With early diagnosis, strong lubrication, protective strategies and regular specialist follow-up, many children maintain good vision. The biggest risk is unrecognized corneal damage, so prompt attention to redness, pain or vision changes is crucial. [91]

4. Why are teeth so affected by dry mouth?
Saliva normally washes away food, buffers acids and contains minerals and antimicrobial factors. Without saliva, acids and bacteria are in constant contact with teeth, quickly causing decay, sensitivity and fractures. That is why fluoride, diet control and frequent dental care are essential. [92]

5. Do pilocarpine or cevimeline work if glands are totally absent?
These drugs stimulate existing gland tissue. If major glands are completely absent, benefit may be limited, but minor glands or residual tissue sometimes respond. Decision to try them depends on imaging, symptom severity and side-effect risk, and should be made by a specialist. [93]

6. Are cyclosporine and lifitegrast safe for long-term use?
Clinical trials and post-marketing data support long-term use of these eye drops for dry eye disease, with most side effects being local (burning, irritation). Systemic absorption is very low. Regular monitoring by an eye doctor is still recommended. [94]

7. Will omega-3 supplements definitely help my dry eye?
Some randomized trials show improvement in symptoms and tear stability, while others show limited benefit. Omega-3s can be tried as an adjunct, especially if diet is low in oily fish, but they should not replace core lubricants or medical treatments. [95]

8. Are stem-cell therapies widely available now?
No. MSC injections and exosome eye drops for dry eye are still in clinical trials. They look promising for immune modulation and tissue repair but are not routine treatments. Anyone offering “stem-cell cures” outside regulated research should be viewed with caution. [96]

9. Can vitamin A supplements alone fix my eye dryness?
Vitamin A supplementation can reverse xerophthalmia caused by deficiency, but in congenital gland aplasia the glands never formed, so lubrication and other therapies are still needed. Excess vitamin A can be toxic, so supplements must be supervised by a doctor. [97]

10. Is it safe to use eye drops many times a day?
Preservative-free lubricating drops are generally safe for very frequent use. Multi-dose bottles with preservatives can irritate the surface if used too often. Your ophthalmologist can suggest specific products and schedules that are safe for long-term, intensive use. [98]

11. Why do I still have pain even when the eye looks okay?
Some people develop neuropathic eye pain, where nerves remain over-sensitive after long-term dryness or injury. In this case, surface treatments may not fully relieve pain, and nerve-focused pain medicines or therapies may be needed, under specialist care. [99]

12. Can I wear contact lenses?
Standard soft contact lenses are often poorly tolerated because they need a healthy tear film. Scleral lenses are usually better because they protect and hydrate the cornea. Lens fitting must be done by an experienced specialist, with careful hygiene to prevent infection. [100]

13. Is this condition life-threatening?
The condition mainly affects eyes and oral health and is not usually life-threatening. However, untreated corneal ulcers can threaten vision, and severe dental disease can affect nutrition and overall health. Good multidisciplinary care keeps these risks low. [101]

14. Can children with ALSG play sports and live normal lives?
With protective eyewear, regular hydration and medical follow-up, most children can attend school, play sports and lead active lives. The main limitations are avoiding eye trauma and maintaining strict oral and eye care routines. [102]

15. Who should coordinate my care?
Ideally, an ophthalmologist, dentist and primary-care doctor work together, with input from genetics or rheumatology if a broader syndrome is present. Because this is a rare condition, referral to centers experienced in complex dry eye and xerostomia can be very helpful. [103]

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: March 05, 2025.